成人发病的散发性共济失调症临床诊疗.pdfVIP

中国临床神经科学 2015 年第 23 卷第4 期 Chin J Clin Neurosci 2015, 23 (4), 459~464，469 ·459 · 进 展 张颖冬 (南京医科大学附属南京医院神经内科 210006) 关键词　共济失调；成人发病；散发性；诊断 摘要 成人发病的散发性共济失调症是由多种原因所致，临床表现以共济失调为主要的多种疾病。致病原因包括中毒、 免疫介导性、维生素缺乏、感染性疾病、变性病以及遗传性疾患等。散发性共济失调临床谱具有明显异质性，临床 正确诊断极具挑战性。但相比于遗传性共济失调症，此类疾患多数治疗效果较好，及时、正确诊断尤为重要。现代 生化、免疫以及影像学技术有助于此类疾患的认识和诊治。文中综述并讨论可致成人发病的散发性共济失调的不同 疾病分类，并着重其临床和神经影像学表现及诊断标准。 Clinical Insights on Adult-onset Sporadic Ataxias ZHANG Ying-dong Department of Neurology, Nanjing First Hospital, Nanjing Medical University, Nanjing 210006, China KEY WORDS　ataxia; adult-onset; sporadic; diagnosis ABSTRACT　Adult-onset sporadic ataxias are a group of disorders manifested predominantly with ataxia, due to a variety of the potential causes including intoxication, immune-mediation, vitamins deficiencies, infections, neurodegeneration, and even some hereditary conditions. The clinical spectrum of these disorders is diverse, and their diagnosis remains a challenge. Compared with hereditary ataxias, most ataxias in this group respond the treatment well, that means the significance of timely and correct diagnosis. The advanced biochemical, immune and imaging tools will help the perception and management on those ataxias. Different categories of adult-onset sporadic ataxia were elucidated with the highlight on their clinical features, neuro-imagingand diagnostic criteria. 共济失调是指肌力正常情况下出现的运动协调 性遗传性共济失调、常染色体隐性遗传性共济失调、 障碍，临床表现为肢体随意运动的幅度及协调发生紊 先天性共济失调、线粒体性共济失调、X-连锁小脑性共 [2] 乱，不能维持躯体姿势和平衡，主要由小脑和(或)与小 济失调和散发性共济失调。也有将此分类简化分为 ： 脑联系纤维结构(特别是脑干)受累病损所致。以共济失 获得性共济失调(指外源性或外源性非遗传原因) 、遗 调为主要或唯一临床表现的共济失调